DESCRIPTION, OVERALL (provided by applicant): The principal goal of the Center for Polycystic Kidney Disease Research at Yale is to facilitate translational research that will advance the understanding and treatment of polycystic kidney diseases. The Center will provide investigators both at Yale and across the country with access to highly specialized services and research tools not otherwise routinely available to support their research. Three Center Cores will be established whose specific objectives are to 1) generate, maintain and provide small animal models of polycystic kidney disease, 2) provide detailed characterizations of the proteomic profiles of urine specimens derived from these mouse models during the course both of disease progression and of experimental therapies, and 3) offer polycystic kidney disease researchers access to advanced physiological tools, including characterization of ion channel activities and state of the art imaging facilities, with which to explore the cellular and molecular basis of polycystic kidney disease. We have identified a research user base of 31 investigators, including 11 at outside institutions, who have expressed specific interest in using core services of the Polycystic Kidney Disease Center at Yale. A Pilot and Feasibility Program will be established to provide initial project funding for young investigators, to attract new investigators into the field of polycystic kidney disease research, and to foster basic and translational studies directly related to polycystic kidney diseases. In addition, an Enrichment Program will be established to promote interdisciplinary interactions and collaborations among investigators participating in the Center;facilitate the application of new technologies to polycystic kidney disease research;and provide an online index of resources developed in the Center (e.g. mouse and zebrafish models and urine proteomes) so that they can be made available to polycystic kidney disease investigators both at Yale and at other institutions. The Center will also include a Research Training Program to enhance the training of graduate students, medical students, and postdoctoral fellows in polycystic kidney disease research in general, and in the specialized models and methodologies provided by the Center Cores in particular. Public Health Relevance: Polycystic kidney disease affects more than 1 in 1,000 individuals. It is the leading genetic cause of renal failure, and 50% of polycystic kidney disease patients will require renal replacement therapy. The Center for Polycystic Kidney Disease Research at Yale will enhance polycystic kidney disease research both at Yale and across the country by offering investigators access to highly specialized services and research tools.